Scoliosis is a 3-dimensional deformity of the spine and trunk. It is typically identified by a lateral curvature of the spine with a Cobb angle >10 degrees as measured on full-length standing PA and lateral radiograph. 80% of all pediatric scoliosis is termed “idiopathic” or of unknown etiology.1, 2
Although several factors have been proposed, including: genetics, connective tissue abnormalities, and associated neurological, muscular and skeletal disorders, its exact etiology remains unclear. It is a diagnosis of exclusion and its evaluation is consistent with a normal neurological exam and lack of anomalous vertebrae on imaging studies.3
Epidemiology including risk factors and primary prevention
The prevalence of idiopathic scoliosis (IS) in the general population is approximately 1.9%- 3% for curves >10 degrees and 0.3% for curves >20 degrees. 1,2 It is stratified into three sub-types:
- Infantile (< 3 years): <1% of diagnoses. M:F (3:2).
- Juvenile (4 to 9 years): 12 – 21% of diagnoses. F:M (1.6-4.4:1.0); ratio increases with increasing age of onset.
- Adolescent (10 to 18 years) (AIS): 80 – 90% of diagnoses. F:M (5:1)
- Cobb angle ≥10°: 3%; F:M -> 2.4:1
- Cobb angle ≥20°: 0.5%; F:M -> 5.4:1
- Cobb angle ≥30°: 0.3%; F:M -> 10:1
- Cobb angle ≥40°: ≤0.1%
F = females; M = males.
Risk factors: family history, joint hypermobility, growth-related factors (increased height and growth velocity), low body mass index (BMI), and female with delayed puberty and late menarche.4
Spinal stability is derived from bone, connective tissue, muscle, and their interaction with the nervous system. Dysfunction in any of these elements leads to imbalance and, ultimately, to deformity. This deformity may manifest as a curvature in the anterior-posterior plane, angulation in the sagittal plane, and/or rotation in the transverse plane.
It is a complex and multifactorial condition. The pathogenesis remains limited.
Disease progression including natural history, disease phases or stages, disease trajectory (clinical features and presentation over time):
Infantile: Most tend to spontaneously resolve.
Juvenile (JIS): Slow progression until ~10 years of age when curve progression is more rapid, coinciding with the period of accelerated spinal growth; more likely to have curves that progress to severe deformity and less likely to respond to a nonsurgical treatment such as bracing.
Adolescent (AIS): The risk of progression depends on skeletal maturity, sex, and curve magnitude. Patients with curves >20 degrees who are skeletally immature are at a greater risk for curve progression.1 Potential for progression is much lower after the completion of spinal growth. However a curve >50 degrees can progress to adulthood and lead to secondary health problems and reduction in quality of life.2, 5
Specific secondary or associated conditions and complications
Severe scoliosis can lead to skin breakdown (ribs overlapping pelvis), poor seating biomechanics, aesthetic concerns and restrictive lung disease. An increased risk of morbidity and mortality may be seen in younger patients (<10 years old) who present with a compromised pulmonary status.
Spinal deformity may lead to chronic back pain in adulthood.2
Essentials of Assessment
Keys in the history include age of onset of the spinal deformity and any observed progression. The usual presenting complaint is chest wall or back asymmetry, though about 25% will present with non-specific pain.
Rapid development of a severe curve suggests a non-idiopathic type of scoliosis. Weakness, sensory changes, gait abnormalities, radiating pain, bowel or bladder dysfunction and other neurologic symptoms suggest an intraspinal pathology such as syringomyelia, tethered cord, dysraphism and spinal cord tumor, etc.6
History of difficulty breathing may suggest pulmonary compromise and is an indication for further evaluation.
Monitor for peak growth velocity by assessing pubertal development and plotting heights on a growth chart.
Look for asymmetry of limb length (curves caused by a leg-length discrepancy should disappear with sitting), shoulder height differences, scapular positioning, pelvic tilt, truncal shift and chest cage deviation. Check for joint deformity and excessive laxity that can be associated with Ehlers-Danlos syndromes, Marfan syndrome, or osteogenesis imperfecta. Foot deformities (cavovarus) may suggest neural axis abnormalities or neuromuscular diseases such as hereditary motor sensory neuropathy or Friedreich ataxia. 7
Perform Adam’s forward-bend test by having the patient bend forward at the waist with knees straight. Asymmetry is evidenced by rib prominence and/or lumbar paraspinal elevation.
A scoliometer measures rotational deformity on the Adam’s forward-bend test, and a trunk rotation of >7 degrees (correlates with >20-degree coronal plane curve) is an indication for further workup. Modern scoliometers are now readily available as applications on smart phones.
Neurologic exam includes motor strength, sensation, reflexes, gait and tandem walk, as well as tests for abdominal reflexes, clonus, Hoffman and Babinski signs.
Visually inspect for skin markings such as sacral dimple, hair tufts, hemangiomas, and café au lait spots to rule out other causes related to scoliosis.
Clinical functional assessment: mobility, self care cognition/behave or/affective state
Activities of daily living (ADLs), Functional Independence Measure (FIM), and WeeFIM measures can be helpful in assessing function (self-care, sitting balance, breathing, feeding, transfers, mobility, ambulation, bowel and bladder management, etc.). Cognition is not affected by scoliosis. Some patients may become power wheelchair users due to mechanical or cardiopulmonary limitations.
Unnecessary except in work-up for metabolic conditions.
Initial radiographic evaluation of patients is with a posterior-anterior (PA) and lateral upright image of the whole spine. The curve magnitude is evaluated using the Cobb method of measuring the angular deformity from the upper end vertebrae to the lower end vertebrae. >10-degrees is defined as scoliosis with an intra-interobserver error of 3-5 degrees. On the lateral radiograph, there is generally an apical hypokyphosis associated with idiopathic scoliosis.1 Scoliosis with increased thoracic kyphosis can be associated with Arnold-Chiari malformation or syringomyelia. 7
Examine for features of skeletal maturity, including the Risser sign (stepwise maturation of the iliac apophysis from anterolateral to posteromedial along the iliac crest) and open versus closed triradiate cartilage of the acetabulum.
The Risser grades are as follows:
- 0 – No ossification
- 1 – Up to 25 percent ossification
- 2 – 26 to 50 percent ossification
- 3 – 51 to 75 percent ossification
- 4 – Greater than 76 percent ossification
- 5 – Full bony fusion of the apophysis
MRI is indicated in children <10 years old with a curve >20-degrees, even in the absence of neurologic symptoms. Consider MRI for congenital scoliosis, abnormal neurologic exam, musculoskeletal deformity such as cavus feet, signs associated with an underlying neural tube defect, or left thoracic curvature with rapid progression.
Supplemental assessment tools
Obtain pulmonary function tests (PFTs) for those with severe curves causing restrictive lung disease. Moderate curves can benefit from baseline PFTs due to risk of pulmonary compromise. CT scan can evaluate a 3-dimensioinal lung volume in young patients who may not be able to comply with pulmonary function testing.
Supine bending and stretch radiographs can be used to assess curve flexibility.
Early prediction of outcomes
Factor determinants of a higher risk of scoliosis progression include: positive family history, laxity of skin and joints (connective tissue defect), flattening of physiological thoracic kyphosis (impedes efficient bracing), angle of trunk rotation exceeding 10, and growth spurt.2
There are no identifiable environmental factors that influence the condition of pediatric idiopathic scoliosis.
Social role and social support system
Psychosocial history is important in assessing adolescents with scoliosis, as a cosmetic deformity can have a negative psychological impact. It is important to consult a neuropsychologist or social worker to address the emotional concerns of the patient and family.
(Identify and/or consider issues relevant to this topic in the areas of ethics, quality of life, professionalism and safety.)
Adolescence is a phase of development during which establishment of self-respect and self-confidence occurs. Although pediatric idiopathic scoliosis is usually asymptomatic, it may make this process challenging and difficult. It can lead to back pain, respiratory problems, and changes in physical appearance, thus negatively impacting quality of life.8
Screening for AIS is controversial; however, the Scoliosis Research Society, the Pediatric Orthopedic Society of North America, the American Academy of Orthopedic Surgeons, and the American Academy of Pediatrics agree that girls should be screened twice, at 10 and 12 years of age, whereas boys should be screened once either at 13 or 14 years of age.1, 2, 9
Rehabilitation Management and Treatments
Available or current treatment guidelines
2016 guidelines from The International Scientific Society on Scoliosis Orthopaedic and Rehabilitation Treatment (SOSORT) provide current evidence and recommendations on conservative treatment (braces and exercise) of idiopathic scoliosis.2
The aims of conservative treatment of pediatric scoliosis are to prevent curve progression with a goal of <50 degrees at skeletal maturity, prevent or treat respiratory dysfunction or spinal pain syndromes and improve aesthetic via postural correction. 1,2 Current management of scoliosis includes observation, bracing, surgery, and the choice of treatment is based on the location and type of the curve, the degree of curvature, and bone maturity.1
At different disease stages.
The management is guided by curve magnitude and remaining skeletal growth.
In patients with Cobb angle <30 degrees and Risser grade 0 to 2, observation and physiotherapeutic scoliosis-specific exercises (PSSEs) are recommended. 2, 9, 10, In patients with Cobb angles of 30 to 45 degrees and Risser grade 0 to 2, in addition to PSSEs, bracing is suggested and should be continued until skeletal maturity.11
Weinstein et al. demonstrated in the BRAIST trial (NEJM, 2013) that bracing significantly decreases the progression of high-risk curves to the threshold for surgery (50 degrees), and longer hours of brace wearing increases the efficacy of bracing, with 13 hours or more being adequate.2,11 Other controlled prospective studies suggest a benefit of bracing on slowing of curvature progression.9
Thoracolumbosacral orthosis (TLSO) is the most commonly used and well tolerated rigid brace. The Rigo Chêneau orthosis (RCO) (Figures 1, 2) was developed with the intent to combine biomechanical forces in three dimensions, including curve de-rotation. RCOs are different from traditional TLSOs in their use of an open pelvis design with anterior openings. Instead of focusing on one-dimensional correction like TLSO, RCO particularly influence on curve de-rotation and treats scoliosis in all three dimensions.12 A study found that the apical vertebral rotation was significantly reduced by the RCO when compared with the TLSO.5
Other bracing options include night-wearing braces (Charleston and the Providence) and nonrigid braces (Spine Cor). A more constricting brace such as Milwaukee with under chin extension is less well tolerated and subsequently no longer plays a role in modern AIS bracing techniques.5, 9,13, 14 A cast is considered a standard first approach in infantile scoliosis, followed by rigid brace.2
There is strong evidence supporting the use of scoliosis specific PT and exercise such as Schroth, and SEAS (Scientific Exercises Approach to Scoliosis) for AIS. PSSEs consist of auto-correction in 3D (defined as the ability to decrease the spinal deformity via active postural realignment of the spine in three dimensions), training in ADLs, stabilizing a corrected posture, and patient education. Therapy may be used alone or in conjunction with braces in patients with any curves of less than 45 degrees.2, 15
Although some randomized trials suggest that PSSEs may reduce the Cobb angle and improve health-related quality of life, 14 systematic reviews have concluded that the beneficial evidence is insufficient.7, 16, 17
Surgical treatment is reserved for patients who fail conservative measures, progress to a Cobb angle >45 degrees in those with skeletal immaturity, and skeletally mature patients with a Cobb angle >50 degrees. 1, 10 Surgery to correct scoliosis involves spinal fusion. Posterior spinal fusion (PSF) with instrumentation is the most common surgical procedure for AIS. 18 A new growth modulation technique (e.g., vertebral body stapling or tethering) utilizes patient’s growth to achieve progressive curve correction by slowing growth on the convex side of the curve. It is a promising surgical treatment of AIS which maintains spinal motion.5, 7
Coordination of care
(Recommend useful team approaches or models of care relevant to this topic.)
Interdisciplinary discussion among pediatrician, physiatrist, and orthopedic surgeon should be held to determine optimal treatment. For patients with progressive deformity, perioperative/postoperative intensive rehabilitation is important. To ensure as a safe home discharge after PSF, coordinated effort of medical, nursing, and rehabilitation providers is required.
Patient & family education
The patient and their family should be educated on the nature and progression of idiopathic scoliosis. They should be aware that treatment is individualized, based on the disease severity and potential for growth. Different treatment options along with their risks and benefits should be thoroughly explained.
Measurement of Treatment Outcomes including those that are impairment-based, activity participation-based and environmentally-based.
Several factors have been studied to examine the treatment outcome of AIS. One of them is curve progression. 1 It is estimated that the curve tends to progress one degree per year after skeletal maturity if it is greater than 50 degrees at the end of growth, in contrast to no curve progression if the curve is less than 30 degrees at the end of growth. 1, 11
Other measurements of treatment outcome include back pain, self-image, sagittal plane evaluation and quality of life assessment (HRQoL). A series of instruments (questionnaires) for post spinal surgery patients have been proposed to evaluate quality of life.2
Translation into Practice: practice “pearls”/performance improvement in practice (PIPs)/changes in clinical practice behaviors and skills
Early diagnosis and monitoring is vital in the treatment of idiopathic scoliosis. The degree of curvature and skeletal maturity will dictate treatment which will range from observation to bracing to surgery.
Prospective evidence now supports the benefit of bracing and PSSEs in the treatment of AIS. 2,12
Cutting Edge/Emerging and Unique Concepts and Practice
Dynamic surface topography (DST) is a rasterstereography based on imaging system designed to evaluate spinal deformity, providing radiation-free imaging of the position, rotation, and shape of the trunk during the gait cycle. Imaging of DST is based on optical acquisition of the entire torso surface. The test aims to decrease the cumulative exposure to x-ray radiation. It is useful in patient monitoring as an alternative to radiography, without diminishing the standard of care.2
Sagittal spine balance of the spine and pelvis in AIS has become very important recently, involving proper adjustment of each spine curvature in the sagittal plane. It involves a harmonious dynamic relationship between the cranium, spine, and pelvis. The analysis of sagittal balance requires radiographic anteroposterior and lateral views of the entire spine in standing and relaxed positions, from C2 to the femoral heads. Overall assessment of balance can be performed using different parameters such as C7 plumb line (sagittal vertical line), pelvis tilt and incidence, and odntoid hip axis angle. Sagittal imbalance is a complex issue that results as a consequence of various etiologies of spinal deformity including AIS. The analysis will guide surgical treatment to allow a compensated spine balance. 19, 20
Gaps in the Evidence-Based Knowledge
Studies on long-term outcomes such as pulmonary disorders, disability, back pain, psychological effects, cosmetic issues, and quality of life are needed and should stratify results by degree of spinal curvature at diagnosis and at skeletal maturity.9
There is also need for developing the questionnaire specifically for measuring quality of life (QoL) in patients treated conservatively, allowing comparisons of QoL between patients treated differently.2
- El-Hawary R, Chukwunyerenwa C. Update on Evaluation and Treatment of Scoliosis. Pediatric Clinics of North America. Vol 61, issue 6, Dec 2014. Pages 1223-1241.
- Negrini S, Donzelli S, Aulisa AG,et al. 2016 SOSORT guidelines: Orthopaedic and Rehabilitation Treatment of Idiopathic Scoliosis during Growth. Scoliosis Spinal Disord. 2018; 13:3. Epub 2018 Jan 10.
- Zaydman AM, Strokova EL, Kiseleva EV, et al. A New Look at Etiological Factors of Idiopathic Scoliosis: Neural Crest Cells. International Journal of Medical Sciences. 2018;15(5):436-446. doi:10.7150/ijms.22894.
- Fadzan M, Bettany-Saltikov J. Etiological Theories of Adolescent Idiopathic Scoliosis: Past and Present. Open Orthop J. 2017 Dec 29;11:1466-1489. doi:10.2174/1874325001711011466. eCollection 2017.
- Addai D, Zarkos J, Bowey AJ. Current concepts in the diagnosis and management of adolescent idiopathic scoliosis. Childs Nerv Syst. 2020 Jun;36(6):1111-1119.
- Newton PO, Wenger DR.Idiopathic scoliosis.In: Morrissy RT,Weinstein SL, eds.Lovell and Winters Pediatric Orthopaedics. 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2006:695-761.
- Scherl S. Adolescent idiopathic scoliosis: Clinical features, evaluation, and diagnosis. https://www.uptodate.com/contents/adolescent-idiopathic-scoliosis-clinical-features-evaluation-and diagnosis?search=idiopathic%20scoliosis&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1. Accessed 7/2021,
- Meng Z-D, Li T-P, Xie X-H, Luo C, Lian X-Y, Wang Z-Y. Quality of life in adolescent patients with idiopathic scoliosis after brace treatment: A meta-analysis. Sharma. VP, ed. Medicine. 2017;96(19):e6828. doi:10.1097/MD.0000000000006828.
- Dunn J, Henrikson NB, Morrison CC, et al. Screening for Adolescent Idiopathic Scoliosis: US Preventive Services Task Force Recommendation Statement. JAMA. 2018 Jan 9;319(2):165-172. doi: 10.1001/jama.2017.19342.
- Ramirez JM, Eberson CP. The Role of Rehabilitation in the Management of Adolescent Idiopathic Scoliosis. R I Med J (2013). 2017 Nov 1;100(11):22-25. PMID: 29088570.
- Weinstein SL, Dolan LA, Wright JG, Dobbs MB. Effects of bracing in adolescents with idiopathic scoliosis. N Engl J Med. 2013 Oct 17; 369(16):1512-21.
- Minsk MK, Venuti KD, Daumit GL, Sponseller PD. Effectiveness of the Rigo Chêneau versus Boston-style orthoses for adolescent idiopathic scoliosis: a retrospective study. Scoliosis Spinal Disord. 2017 Mar 20;12:7.
- Gammon SR, Mehlman CT, Chan W, et al. A comparison of thoracolumbosacral orthoses and SpineCor treatment of adolescent idiopathic scoliosis patients using the Scoliosis Research Society standardized criteria. J Pediatr Orthop 2010; 30:531.
- Janicki JA, Poe-Kochert C, Armstrong DG, Thompson GH. A comparison of the thoracolumbosacral orthoses and providence orthosis in the treatment of adolescent idiopathic scoliosis: results using the new SRS inclusion and assessment criteria for bracing studies. J Pediatr Orthop 2007; 27:369.
- Schreiber, S., Parent, E., KhodayariMoez, E., Hedden, D., Hill, D., Moreau, M. et al. Schroth physiotherapeutic scoliosis-specific exercises added to the standard of care lead to better Cobb angle outcomes in adolescents with idiopathic scoliosis – An assessor and statistician blinded randomized controlled trial. PLoS One 2016 ;11(12), 1–17.
- Day JM, Fletcher J, Coghlan M, Ravine T. Review of scoliosis-specific exercise methods used to correct adolescent idiopathic scoliosis. Arch Physiother. 2019 Aug 23;9:8.
- Fan Y, Ren Q, To MKT, Cheung JPY. Effectiveness of scoliosis-specific exercises for alleviating adolescent idiopathic scoliosis: a systemic review. BMC Musculoskelet Dissord. 2020;21(1):495. Epub 2020 Jul 27
- Geck MJ, Rinella A, Hawthorne D, et al. Comparison of surgical treatment in Lenke 5C adolescent idiopathic scoliosis: anterior dual rod versus posterior pedicle fixation surgery: a comparison of two practices. Spine (Phila Pa 1976) 2009; 34:1942.
- Kim D, Davis DD, Menger RP. Spine Sagittal Balance. [Updated 2021 Aug 11]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-.
- Le Huec JC, Thompson W, Mohsinaly Y, et al.Sagittal balance of the spine. Eur Spine J. 2019 Sep;28(9):1889-1905.
Original Version of the Topic
Yuxi Chen, MD, Matthew Oglesby, MD, Nan Wang, MD. Pediatric Idiopathic Scoliosis. 5/29/2018
Yuxi Chen, MD
Ipsen; Research Grant paid to institution; PI for Pediatric lower limb spasticity study and Adult lower limb spasticity study
MERZ; Payment; Advisory board
Andrew Bloomfield, MD
Nothing to Disclose
Nahyun Kim, MD
Nothing to Disclose